Table 2.
Characteristics of patients diagnosed with autoimmune CNS disorder
| No. Age/ sex | Onset and neurological presentation | MRI | CSF | EEG | PET metabolism | AE criteria |
|---|---|---|---|---|---|---|
| 1. 30 M | <3 m Memory loss, behaviour change and hemiparesis (S-L) | - | – | – | SPECT: Global hypoperfusion | Prob HE |
| 2. 53 F | <3 m Memory loss, aphasia, hemiparesis (S-L), loss of motivation and psychosis | T2-H frontal (B) Gad+ | – | DS | NA | Poss AE |
| 3. 48 F | <3 m Memory loss, disorientation and hypersomnia | – | OCB+ | – | NA | Prob HE |
| 4. 16 F | Unclear onset memory loss, hypersomnia, hallucinations and seizures | - | WBC 12 | SW(T) | - | Prob HE* |
| 5. 45 F | <3 m Confusion, hemiparesis and aphasia (S-L) | - | WBC 25 | NA | NA | Prob HE |
| 6. 41 M | <3 m Memory loss, confusion and behaviour change | - | – | NA | NA | Prob HE |
| 7. 53 M | <3 m Memory, executive and visuospatial difficulties, behaviour change | - | – | - | Global hypo | Prob HE |
| 8. 32 M | <3 m Confusion, hemiparesis and aphasia (S-L), combative | - | – | - | NA | Def AE |
| 9. 50 M | <3 m Amnesia, aphasia (S-L), seizures, agitation, psychosis and hyper-religiosity | - | – | - | NA | Prob HE |
| 10. 33 F | <3 m Memory, confusion, emotional lability, hallucinations and myoclonus | - | – | - | – | Prob HE |
| 11. 44 M | <3 m Confusion, speech difficulty and facial weakness | - | WBC 15 | - | – | Prob HE |
| 12. 83 M | <3 m Confusion and hemiparesis (S-L) | - | – | - | NA | Prob HE |
| 13. 26 F | <3 m Disorientation, psychosis and seizure | - | – | NA | NA | Prob HE |
| 14. 63 M | <3 m Confusion, hemiparesis and aphasia (S-L) | - | – | DS | NA | Prob HE |
| 15. 42 F | <3 m Confusion and hallucinations | - | – | – | NA | Prob HE |
| 16. 13 M | <3 m Seizures and memory loss | - | – | SW(F) | NA | Prob HE |
| 17. 67 F | <3 m Disorientation, confabulation, personality change, agitation and hallucinations | - | – | - | Focal (FT)hypo | Prob HE |
| 18. 70 F | <3 m Disorientation and memory loss | - | – | – | NA | Def AE |
| 19. 87 F | <3 m Disorientation, myoclonus and hypersomnia | - | – | SW(T) | Focal (FTP) hypo and hyper (T) | Prob HE |
| 20. 42 F | <3 m Memory loss and hypersomnia | - | – | - | NA | Prob HE |
| 21. 69 F | 1–2 y memory loss 6 m more rapid decline | - | IgGs | – | Focal (PT) hypo | Nonea |
| 22. 73 F | <3 m Confusion, behaviour change and seizures | Hippo atrophy & T2-H (R) | WBC 7 | SZ | Focal (P) hypo and hyper (T) | Prob AE |
| 23. 41 F | <3 m Confusion and emotional lability | - | – | – | NA | Prob HE |
| 24. 30 F | 6 m Seizures and memory loss | - | WBC 7 | SW(P) | NA | Prob HE* |
| 25. 43 M | <3 m Aphasia, memory loss, seizures and hemiparesis (S-L) | - | WBC 25 OCB+ IgGi | SW(FT), TIRDA | NA | Prob HE |
| 26. 70 M | <3 m Memory loss, disorientation and seizures | T2-H temporal (B) | – | SZ (T) | – | Def LE |
| 27. 56 M | <3 m Confusion, combative, hallucinations and cranial neuropathies (VII, IX, X) | - | – | NA | NA | Prob HE |
| 28. 67 F | <3 m Confusion, behaviour change, mania and psychosis | - | – | DS | Focal (F)hypo | Prob HE |
| 29. 51 F | <3 m Memory loss, psychosis and mania | - | – | – | – | Prob HE |
| 30. 66 M | <3 m Confusion, agitation, hallucinations and ataxia | Frontal T2-H (R), Gad+ (dural) | WBC 8 | GPEDs, DS | NA | Prob AE |
| 31. 42 F | <3 m Confusion, delusions and seizures | Hippo atrophy and T2-H (L) | WBC 8 | – | NA | Prob AE |
| 32. 41 F | Months memory loss, hemiparesis and ataxia | - | OCB+ | NA | Global hypo | Prob HE* |
| 33. 44 F | <3 m Confusion, ataxia and dysarthria | Brainstem atrophy | IgGi | – | NA | Prob AE |
| 34. 42 M | 5-y Memory loss, aphasia, apraxia and tremor | Frontotemporal T2-H (L) | WBC 12 OCB+ IgGi | – | NA | Nonea |
| 35. 68 F | <3 m Memory loss, aphasia, hallucinations and ataxia | - | – | SW(T), TIRDA | NA | Prob HE |
| 36. 56 F | <3 m Memory loss, abnormal movements and right upper extremity | Hippo atrophy & T2-H(B), Gad+ | – | SW (T) | NA | Def LE |
| 37. 62 M | 4 m Memory loss, concentration and neuropathy | – | – | – | NA | Prob HE* |
| 38. 39 F | <3 m Multiple cranial neuropathies | - | – | NA | - | Prob HE |
| 39. 61 F | 8 m New musicogenic seizures | - | – | SW(T), SZ (FT) | NA | Noneb |
+ yes, - normal or non-specific, S-L, stroke-like; m, months; y, years; T2-H, T2-hyperintensity; AE, autoimmune encephalitis; HE, Hashimoto encephalopathy; LE, limbic encephalitis; DS, diffuse slowing; SW, sharp waves; SZ, seizure; TIRDA, temporal intermittent rhythmic delta activity; GPEDs, generalized periodic epileptiform discharges; M, male; F, female; NA, not available; Hippo, hippocampus; F, frontal; T, temporal; P, parietal; FT, fronto-temporal; FTP, fronto-temporo-parietal; PT, parieto-temporal; L, left; R, right; B, bilateral; Gad+, contrast enhancement; IgGi, elevated IgG index; IgGs, elevated IgG synthesis rate; Prob, probable; Poss, possible; Def, definite.
a
Autoimmune dementia.
b
Autoimmune epilepsy.
*
Symptom onset excluded.